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. 2016 Jul 27:16:542.
doi: 10.1186/s12885-016-2513-9.

Treatment and outcome of Ganglioneuroma and Ganglioneuroblastoma intermixed

Boris Decarolis  1 , Thorsten Simon  1 , Barbara Krug  2 , Ivo Leuschner  3 , Christian Vokuhl  3 , Peter Kaatsch  4 , Dietrich von Schweinitz  5 , Thomas Klingebiel  6 , Ingo Mueller  7 , Lothar Schweigerer  8 , Frank Berthold  1 , Barbara Hero  9
Affiliations

Treatment and outcome of Ganglioneuroma and Ganglioneuroblastoma intermixed

Boris Decarolis et al. BMC Cancer. .

Abstract

Background: Ganglioneuroma (GN) and ganglioneuroblastoma intermixed (GNBI) are mature variants of neuroblastic tumors (NT). It is still discussed whether incomplete resection of GN/GNBI impairs the outcome of patients.

Methods: Clinical characteristics and outcome of localized GN/GNBI were retrospectively compared to localized neuroblastoma (NB) and ganglioneuroblastoma-nodular (GNBN) registered in the German neuroblastoma trials between 2000 and 2010.

Results: Of 808 consecutive localized NT, 162 (20 %) were classified as GN and 55 (7 %) as GNBI. GN/GNBI patients presented more often with stage 1 disease (68 % vs. 37 %, p < 0.001), less frequently with adrenal tumors (31 % vs. 43 %, p = 0.001) and positive mIBG-uptake (34 % vs. 90 %, p < 0.001), and had less often elevated urine catecholamine metabolites (homovanillic acid 39 % vs. 62 %, p < 0.001, vanillylmandelic acid 27 % vs. 64 %, p < 0.001). Median age at diagnosis increased with grade of differentiation (NB/GNBN: 9; GNBI: 61; GN-maturing: 71; GN-mature: 125 months, p < 0.001). Complete tumor resection was achieved at diagnosis in 70 % of 162 GN and 67 % of 55 GNBI, and after 4 to 32 months of observation in 4 GN (2 %) and 5 GNBI (9 %). Eleven patients received chemotherapy without substantial effect. Fifty-five residual tumors (42 GN, 13 GNBI) are currently under observation (median: 44 months). Five patients (3 GN, 2 GNBI) showed local progression; all had tumor residuals > 2 cm. No progression occurred after subtotal resection. Two patients died of treatment, none of tumor progression.

Conclusions: GN/GNBI account for one quarter of localized NT and differ from immature tumors in their clinical features. Chemotherapy is not effective. Subtotal resection appears to be a sufficient treatment.

Trial registration: ClinicalTrials.gov identifiers - NB97 (NCT00017225; registered June 6, 2001); NB2004 (NCT00410631; registered December 11, 2006).

Keywords: Ganglioneuroblastoma intermixed; Ganglioneuroma; Residual tumor; Subtotal resection; Surgery; Therapy; Treatment.

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Figures

Fig. 1
Fig. 1
Grade of differentiation in relation to median age at diagnosis. NB = neuroblastoma, GNBN = ganglioneuroblastoma nodular, GNBI = ganglioneuroblastoma intermixed, GN = ganglioneuroma
Fig. 2
Fig. 2
Extent of initial surgeries and size of residual tumors currently under observation. GNBI = ganglioneuroblastoma intermixed, GN = Ganglioneuroma, n.a. = data not available. a) Surgeries of patients with Ganglioneuroma, b) Surgeries of patients with Ganglioneurblastoma
Fig. 3
Fig. 3
Outcome by histology. NB = neuroblastoma, GNBN = ganglioneuroblastoma nodular, GNBI = ganglioneuroblastoma intermixed, GN = ganglioneuroma. a) Event-free survival (EFS) by histology, b) Overall survival (OS) by histology
Fig. 4
Fig. 4
Outcome of GN/GNBI by tumor residuals. GNBI = ganglioneuroblastoma intermixed, GN = ganglioneuroma. a)EFS for GN/GNBI by tumor residuals, b) OS for GN/GNBI by tumor residuals, p = major residuals vs. no or minor residuals
Fig. 5
Fig. 5
Outcome of ganglioneuroma (GN) by tumor residuals. a) EFS for GN by tumor residuals, b) OS for GN by tumor residuals, p = major residuals vs. no or minor residuals
Fig. 6
Fig. 6
Outcome of ganglioneuroblastoma intermixed (GNBI) by tumor residuals. a) EFS for GNBI by tumor residuals, b) OS for GNBI by tumor residuals, p = major residuals vs. no or minor residuals
See this image and copyright information in PMC

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